Interstitial lung disease

Interstitial lung disease (ILD) is a general term for a variety of disorders that cause scarring and damage to the lungs. Scarring is usually progressive –meaning it gets worse over time and can make it difficult for patients to breathe and transfer enough oxygen into the blood. Some cases of ILD are caused by exposure to dangerous substances like asbestos, or can be triggered by autoimmune diseases like rheumatoid arthritis. Most cases, however, have no clear cause and there is no cure for ILD at this time. Doctors will focus on managing the symptoms and complications of ILD and may discuss a lung transplant for advanced cases.

Causes

When an irritant or toxic substance damages the lining of the lungs, usually the tissues know just how to heal as the body works in perfect harmony to repair and replace damaged areas. But with ILD, the healing process is abnormal, and the tissue around the lung’s air sacs (alveoli) becomes scarred and thick. Alveoli resemble tiny clusters of grapes, and are found at the base of the lungs where oxygen passes into the blood stream. If the alveoli are damaged, oxygen cannot pass to the other side.

Medications or drugs: Some types of chemotherapy, heart medications used to treat an abnormal heart rhythms, and a few kinds of antibiotics have been linked to ILD. Just because you take a heart medication or are completing chemo does not mean you will get ILD. Radiation therapy may also trigger ILD but several other factors including length of exposure, underlying medical conditions and concurrent chemotherapy use must also be considered.

Medical conditions: Rheumatoid arthritis, scleroderma, sarcoidosis, and lupus have all been linked to higher rates of ILD. Environmental hazards: ILD is higher in patients who have prolonged exposure to silica dust, asbestos, grain dust, and bird and animal droppings.

Symptoms

The main symptoms of ILD include shortness of breath that gets worse with activity or exertion and a dry cough. By the time symptoms appear, most patients will already have irreversible lung damage. However, this should not delay treatment.

Testing and diagnosis

Identifying a cause of ILD can be challenging, and may not be possible at all. A variety of imaging and lung tests will be needed to rule out other types of lung problems and provide an accurate diagnosis. These include:

Treatment

Because there is no cure for ILD, patients should expect to symptom and complication management. This may include the use of steroids to suppress the immune system and decrease inflammation in and around the lungs. Oxygen therapy may help decrease stress on the heart and body and provide more oxygen for transfer to the blood stream. Pulmonary rehabilitation can be used to learn new breathing techniques and improve endurance. Younger patients may be candidates for a lung transplant as a last resort when all other management options have failed.